Congenital and acquired single-lung patients: long-term follow-up reveals high mortality risk

Purpose

Single-lung patients are uncommon. Case reports suggest that these patients have significant morbidity and mortality risks because of associated anomalies, airway obstruction as well as decreased pulmonary reserve. This study was designed to review morbidity and mortality of single-lung patients.

Methods

A retrospective review of single-lung patients admitted to our hospital from January 1988 to June 2007.

Results

Twelve single-lung patients were treated in our hospital with mean follow-up of 22.9 months. Six congenital lung aplasia patients presented as neonates, with 2 diagnosed antenatally. Of 4 patients with respiratory distress, 3 required surgical interventions to correct airway symptoms. Three patients died before 9 months of age because of respiratory failure, sepsis, or cardiac arrest. The remaining survivors have minimal respiratory distress. There were 6 pneumonectomy patients as follows: 5 for malignancies and 1 for bronchiectasis. There were 2 deaths from recurrent disease. Four patients had respiratory symptoms with one patient requiring tissue expander insertion for the correction of airway torsion from mediastinal shift.

Conclusions

The morbidity and mortality of infant single-lung patients are significant. Respiratory distress from decreased pulmonary reserve must be distinguished from airway obstruction because of mediastinal shift. Bronchoscopic assessment was invaluable to the diagnosis and management of these patients.     

ABO血型不合异基因造血干细胞移植患者ABO血型抗体的监测

目的:监测ABO血型不合造血干细胞移植患者IgM型和IgG型ABO血型抗体的变化,研究这些变化在移植患者血型转变中的作用。方法:用凝聚胺法和微柱凝胶法监测32例移植患者ABO血型,IgM型和IgG型ABO血型抗体的变化。结果:32例ABO血型不合患者血型全部转变为供者型,造血重建时间结果分析表明血型不合对于粒系和血小板的恢复无影响;患者血型转为供者血型后,其血清中缺乏相对应的抗体;32例ABO血型不合患者中有6例供受者移植模式为A→O,其中有3例发生PRCA,且均检出IgG型抗A抗体,其余29例未检测到IgG血型抗体;3例PRCA患者红系造血恢复时间明显延长。结论:IgG型ABO血型抗体可能会抑制ABO血型不合骨髓移植患者的红系分化成熟,在PRCA的发生中可能起重要作用。监测ABO血型不合移植患者体内的ABO血型抗体变化可指导输血治疗,避免溶血性输血反应。     

Pure red cell aplasia due to parvovirus B19 infection after liver transplantation： A case report and review of the literature

Pure red cell aplasia （PRCA） due to parvovirus B19 （PVB19） infectiori after solid organ transplantation has been rarely reported and most of the cases were renal transplant recipients, Few have been described after liver transplantation. Moreover, little information on the management of this easily recurring disease is available at present. We describe the first case of a Chinese liver transplant recipient with PVB19-induced PRCA during immunosuppressive therapy. The patient suffered from progressive anemia with the lowest hemoglobin level of 21 g/L. Bone marrow biopsy showed selectively inhibited erythropoiesis with giant pronormoblasts. Detection of PVB19-DNA in serum with quantitative polymerase chain reaction （PCR） revealed a high level of viral load. After 2 courses of intravenous immunoglobulin （IVIG） therapy, bone marrow erythropoiesis recovered with his hemoglobin level increased to 123 g/L. He had a lowlevel PVB19 load for a 5-too follow-up period without recurrence of PRCA, and finally the virus was cleared. Our case indicates that clearance of PVB19 by IVIG in transplant recipients might be delayed after recovery of anemia.     

A case of pure red cell aplasia and systemic lupus erythematosus caused by human parvovirus B19 infection

Human parvovirus B19 (B19) rarely induces pure red cell aplasia (PRCA) in healthy hosts. Meanwhile B19 infection is often clinically similar to systemic lupus erythematosus (SLE), and several cases have been reported wherein B19 actually stimulated SLE exacerbation in an immunocompetent subject. An 82-year-old healthy woman was diagnosed to have complicated with B19 infection and PRCA. Four weeks later, she had high fever, polyarthritis, and oral ulcers, additionally diagnosed with SLE, and subsequently, 15 mg of prednisone was started. This is the first case wherein B19 infection caused both PRCA and SLE in a healthy patient as far as our investigations are concerned.     

Bone marrow aplasia--a rare complication of imatinib therapy in CML patients

Imatinib mesylate therapy in CML patients is a generally well tolerated without any significant hematological adverse drug effects. However, complications like anemia and cytopenias have been described in literature. A very few case reports of bone marrow aplasia following imatinib therapy have been reported so far. We here report five patients of CML who developed bone marrow aplasia following imatinib therapy.     

Alemtuzumab is safe and effective as immunosuppressive treatment for aplastic anaemia and single‐lineage marrow failure: a pilot study and a survey from the EBMT WPSAA

An alemtuzumab‐based experimental immunosuppressive treatment (IST) regimen was investigated in 35 patients with severe aplastic anaemia (SAA), pure red cell (PRCA) or pure white cell aplasia (PWCA). Alemtuzumab total dose was 73–103 mg s.c., followed by cyclosporine. No serious toxicity due to the regimen was observed. Adverse events were clinically irrelevant; infectious events were rare. The total response rate was 58%, 84% and 100% in SAA, PRCA and PWCA, respectively, with corresponding 6 months cumulative response probabilities of 84%, 84% and 100%. Subcutaneous alemtuzumab is a feasible and sufficiently safe IST regimen for patients suffering from immune‐mediated marrow failures.     

Twenty years of experience with Krzeski's cystovaginoplasty for vaginal agenesis in Mayer-Rokitansky-Küster-Hauser syndrome: anatomical, histological, cytological and functional results

OBJECTIVE

To evaluate the long‐term anatomical results using the original method of vaginal reconstruction with a pedicled bladder flap (Krzeski’s cystovaginoplasty, CVP) in women with Mayer‐Rokitansky‐Küster–Hauser syndrome (MRKHS) and the evaluation of radiological, histological, cytohormonal and functional results of CVP.

PATIENTS AND METHODS

Between 1981 and 2000, 38 patients (mean age 22.5 years, range 18–40) with MRKHS underwent CVP. A physical examination was used to evaluate the anatomical results, 27 patients had vaginography, and biopsies of the neovaginal wall and cytohormonal smears were taken in two. Functional sexual and urinary results, and opinions on CVP, were evaluated by an inventory mailed to 37 patients in 2000.

RESULTS

The anatomical result was good in 37 patients during a mean (range) follow‐up of 9 (0.25–19) years; the result was good in 30 patients, but seven developed vaginal stenosis that was successfully repaired. There were two cases of post‐coital vesicovaginal fistula (VVF) at 18 months after CVP. Vaginal biopsies showed epithelialization of the posterior vaginal wall and gradual metaplastic changes from urothelium to stratified nonsquamous epithelium. Cytological smears showed a normal biphasic pattern and neovaginal susceptibility to hormonal milieu. In all, 27 patients (73%) responded to the questionnaire. All had sexual partners and started sexual intercourse at a mean of 14 months after CVP; 89% experience orgasms and in 48% the vagina was the source; 40% sometimes used lubricants and seven (26%) used vaginal dilators. Lower urinary tract symptoms (LUTS) after CVP were reported by 19 (66%) of the women and in six the LUTS were persistent. One patient was not satisfied with the functional and anatomical result of CVP, 89% declared that it improved sexual life, 93% would undergo CVP again and in 92% the quality of their sexual life was improved. All patients, when asked, stated that they would recommend CVP to another patient with MRKHS.

CONCLUSIONS

Vaginal reconstruction by CVP is characterized by good anatomical and functional results, sustained by long‐term observation. The resultant epithelium is very similar to that of the native vagina in histology and function. All vaginas are functional and the level of patient satisfaction was high. The level of complications was acceptable, but in some patients LUTS can persist.     

Asymmetrical nonscalp aplasia cutis congenita: a case report

Aplasia cutis congenita or congenital absence of the skin is a rare and serious congenital disease; its etiopathogenesis remains unclear. In this condition, localized or widespread areas of skin are absent at birth. A newborn suffering from an unusual aplasia cutis congenita located asymmetrically on the nonscalp, without blistering, was presented. This patient was completely healed with conservative treatment.     

DiGeorge综合征(附2例尸检报告)

目的：报告2例先天性胸腺发育不良病例，以提高对该病的认识。方法：通过尸体解剖，应用组织病理学技术方法，观察胸腺、脾脏、淋巴结及骨髓等组织学改变，选择免疫组化（CD20、CD3、CD5、CD45RA、CD45RO、EBV）和特殊染色进行观察。结果：淋巴器官发育不良，胸腺缺乏皮髓分化；脾组织内淋巴细胞稀疏，脾小体鞘区淋巴细胞少；淋巴结淋巴滤泡缺乏；肠壁、阑尾及咽部粘膜等淋巴组织淋巴细胞稀疏；骨髓造血细胞无明显异常。结论：由于先天性胸腺发育不良，患儿存在原发性免疫缺陷，招致反复难治性感染。如能做出早期正确诊断，对症治疗的同时，可考虑胚胎胸腺移植及胸腺素治疗，以改善患者免疫缺陷状态。